- Risk Factors
- Signs & Symptoms
Childhood Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system.
Childhood Hodgkin lymphoma is a type of cancer that develops in the lymph system, part of the body's immune system. The lymph system is made up of the following:
- Lymph: Colorless, watery fluid that travels through the lymph system and carries white blood cells called lymphocytes. Lymphocytes protect the body against infections and the growth of tumors.
- Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream.
- Lymph nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the underarm, pelvis, neck, abdomen, and groin.
- Spleen: An organ that makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. The spleen is on the left side of the abdomen near the stomach.
- Thymus: An organ in which lymphocytes grow and multiply. The thymus is in the chest behind the breastbone.
- Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils make lymphocytes.
- Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow makes white blood cells, red blood cells, and platelets.
Because lymph tissue is found throughout the body, Hodgkin lymphoma can start in almost any part of the body and spread to almost any tissue or organ in the body.
Lymphomas are divided into two general types: Hodgkin lymphoma and non-Hodgkin lymphoma. (See the PDQ summary on Childhood Non-Hodgkin Lymphoma Treatment for more information.)
Hodgkin lymphoma can occur in both children and adults; however, treatment for children may be different than treatment for adults. (See the PDQ summary on Adult Hodgkin Lymphoma Treatment for more information.)
There are two types of childhood Hodgkin lymphoma.
The two types of childhood Hodgkin lymphoma are:
- Classical Hodgkin lymphoma.
- Nodular lymphocyte-predominant Hodgkin lymphoma.
Classical Hodgkin lymphoma is divided into four subtypes, based on how the cancer cells look under a microscope:
- Lymphocyte-rich classical Hodgkin lymphoma.
- Nodular sclerosis Hodgkin lymphoma.
- Mixed cellularity Hodgkin lymphoma.
- Lymphocyte-depleted Hodgkin lymphoma.
Age, gender, and Epstein-Barr virus infection can affect the risk of developing childhood Hodgkin lymphoma.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for childhood Hodgkin lymphoma include the following:
- Being between the ages of 5 and 14. In children younger than 14 years, it is more common in boys than in girls.
- Being infected with the Epstein-Barr virus.
- Having a brother or sister with Hodgkin lymphoma.
Possible signs of childhood Hodgkin lymphoma include swollen lymph nodes, fever, night sweats, and weight loss.
These and other symptoms may be caused by childhood Hodgkin lymphoma or by other conditions. A doctor should be consulted if any of the following problems occur:
- Painless, swollen lymph nodes in the neck, chest, underarm, or groin.
- Night sweats.
- Weight loss for no known reason.
- Itchy skin.
Tests that examine the lymph system are used to detect (find) and diagnose childhood Hodgkin lymphoma.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
Lymph node biopsy:
The removal of all or part of a lymph node. One of the following types of
biopsies may be done:
- Excisional biopsy: The removal of an entire lymph node.
- Incisional biopsy: The removal of part of a lymph node.
- Core biopsy: The removal of tissue from a lymph node using a wide needle.
- Fine-needle aspiration (FNA) biopsy: The removal of tissue from a lymph node using a thin needle.
- Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
- The number of red blood cells, white blood cells, and platelets.
- The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
- The portion of the blood sample made up of red blood cells
- Sedimentation rate: A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube.
- Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
- Gallium scan: A procedure to detect areas of the body where cells, such as cancer cells, are dividing rapidly. A very small amount of radioactive material, gallium, is injected into a vein and travels through the bloodstream. The gallium collects in the bones or other tissues (organs) and is detected by a scanner.
- Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
- Immunophenotyping: A test in which the cells in a sample of blood or bone marrow are looked at under a microscope to find out the type of malignant (cancerous) lymphocytes that are causing the lymphoma.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
- The stage of the cancer.
- The size of the tumor and how quickly it shrinks after initial treatment.
- The patient's symptoms when diagnosed.
- Certain features of the cancer cells.
- Whether the cancer is newly diagnosed, does not respond to initial treatment, or has recurred (come back).
The treatment options also depend on:
- The child's age and gender.
- The risk of long-term side effects.
Most children and adolescents with newly diagnosed Hodgkin lymphoma can be cured.
After childhood Hodgkin lymphoma has been diagnosed, tests are done to find out if cancer cells have spread within the lymph system or to other parts of the body.
The process used to find out if cancer has spread within the lymph system or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. Treatment is based on the stage and other factors that affect prognosis. The following tests and procedures may be used in the staging process:
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). An MRI of the abdomen and pelvis may be done.
- PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
- Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for abnormal cells.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through tissue. Cancer invades the surrounding normal tissue.
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
The letters "E" and "S" may be used to describe the stages of childhood Hodgkin lymphoma.
- E: Cancer is found in an organ or tissue that is not part of the lymph system but which may be next to an involved area of the lymph system.
- S: Cancer is found in the spleen.
The following stages are used for childhood Hodgkin lymphoma:
Stage I is divided into stage I and stage IE.
- Stage I: Cancer is found in one group of lymph nodes.
- Stage IE: Cancer is found in one group of lymph nodes and has spread to a nearby organ or tissue that is not part of the lymph system.
Stage II is divided into stage II and stage IIE.
- Stage II: Cancer is found in two or more lymph node groups on the same side of the diaphragm.
- Stage IIE: Cancer is found in two or more lymph node groups on the same side of the diaphragm and has spread from one of those lymph nodes to a nearby organ or tissue that is not part of the lymph system.
Stage III is divided into stage III, stage IIIE, stage IIIS, and stage IIIE+S.
- Stage III: Cancer is found in lymph node groups on both sides of the diaphragm.
- Stage IIIE: Cancer is found in lymph node groups on both sides of the diaphragm and has spread from one of these lymph nodes to a nearby organ or tissue that is not part of the lymph system.
- Stage IIIS: Cancer is found in lymph node groups on both sides of the diaphragm and in the spleen.
- Stage IIIE+S: Cancer is found in lymph node groups on both sides of the diaphragm and in the spleen, and has spread from one of these lymph node groups to a nearby organ or tissue that is not part of the lymph system.
In stage IV, cancer is found throughout one or more organs that are not part of the lymph system and may be in lymph nodes that are near or far away from those organs.
Untreated, classical Hodgkin lymphoma is divided into risk groups.
Untreated, classical childhood Hodgkin lymphoma is divided into risk groups based on the bulk of the tumor (tumors that are 5 centimeters or larger are considered "bulky") and whether the patient has "b" symptoms (fever, weight loss, or night sweats). Treatment is based on the risk group.
- Patients with stage I or stage II disease; and
- No bulky tumors or "b" symptoms.
- Patients with stage I or stage II disease, with bulky tumors, or with "b" symptoms; or
- Patients with stage III or stage IV disease without "b" symptoms.
- High-risk disease: Patients with stage III or stage IV disease with "b" symptoms.
Childhood Hodgkin lymphoma is staged again after initial chemotherapy.
A PET or gallium scan is done after chemotherapy ends to find out how well the chemotherapy worked.
Primary Progressive/Recurrent Hodgkin Lymphoma in Children and Adolescents
Primary progressive Hodgkin lymphoma is lymphoma that continues to grow or spread during treatment. Recurrent Hodgkin lymphoma is cancer that has recurred (come back) after it has been treated. The lymphoma may come back in the lymph system or in other parts of the body, such as the lungs, liver, bones, or bone marrow.